Chondrosarcoma

What is a chondrosarcoma?

A chondrosarcoma is a type of rare bone cancer that typically develops in cartilage — the flexible connective tissue that protects your joints and bones. Healthcare providers may call this condition a sarcoma.

This cancer can appear anywhere you have cartilage, but it usually forms in your:

• Arms and shoulder blade.
• Legs.
• Pelvis.
• Ribs.
• Sternum (breastbone).

They may also develop in benign bone tumors that become cancerous.

Chondrosarcomas are very rare, affecting 1 in 200,000 people in the U.S. You can develop a chondrosarcoma at any age, but the condition is more common in people ages 40 to 75, with most people receiving a diagnosis at age 51. The most common treatment is surgery to remove cancerous cartilage and bone.

Types of chondrosarcomas

There are several different types of chondrosarcomas. Chondrosarcomas that develop in cartilage are named for the cells that make up the tumors:

Conventional chondrosarcoma

This is the most common type, accounting for more than 85% of all chondrosarcomas. It typically affects people ages 50 to 70 and develops in your femur, humerus (upper arm bone) and pelvis. Conventional chondrosarcomas grow very slowly and are less likely to spread (metastasize) than other types.

Dedifferentiated chondrosarcoma

Dedifferentiated chondrosarcomas account for 10% of all chondrosarcomas. This type tends to develop in adults aged 60 and older and grows faster than most other chondrosarcomas. The fast-growing tumors usually develop in your humerus, femur or pelvic bones.

They’re known as dedifferentiated chondrosarcomas because some cells start out as typical chondrosarcomas, but then some parts of the tumor change into cells like those of a high-grade sarcoma. High-grade sarcomas are cancers that tend to grow and spread very quickly, including osteosarcoma, fibrosarcoma and undifferentiated pleomorphic sarcoma.

Clear cell chondrosarcoma

This type accounts for 2% of all chondrosarcomas. It typically affects people ages 30 to 50 but can affect people in their 20s. Clear cell chondrosarcomas are typically found near a joint in your arm or leg. They tend to grow slowly and rarely spread to other areas in your body.

Mesenchymal chondrosarcoma

This type often affects adults ages 19 to 30. It typically develops in your spine, ribs or jaw. Mesenchymal chondrosarcoma often grows quickly and is more likely to spread than other chondrosarcomas and come back (recur) after treatment.

Extraskeletal myxoid chondrosarcoma is a form of mesenchymal chondrosarcoma. It forms in the soft tissue in the upper part of your arms and legs but not in your bone or cartilage.

What are the symptoms of chondrosarcoma?

Most chondrosarcomas grow slowly, causing symptoms that take months to develop. The most common symptoms are:

• Bone pain in a specific area of your body that comes and goes and gets worse at night.
• Swollen spot or lump on a bone, like on your arm, leg or ribs.
• Fatigue.
• Unintentional weight loss.

It’s important to remember that bone cancer is rare. Many chondrosarcoma symptoms are like the symptoms of other less serious issues. A lump on your leg may not indicate bone cancer.

But if you have a lump that doesn’t go away within two weeks or you feel exhausted all the time, talk to a healthcare provider. They’ll check your overall health to find out what’s causing your symptoms.

What causes chondrosarcoma?

Researchers don’t know the exact cause but link the condition to genetic disorders, including:

• Li Fraumeni syndrome: People with this condition are likely to develop cancerous tumors, including chondrosarcomas.
• Maffucci syndrome and Ollier’s disease: These disorders cause benign tumors in your cartilage, bone and skin.
• Hereditary multiple osteochondromas: These are multiple benign bone tumors that can disrupt your bone growth.

In some cases, pathologists who study genetic changes in chondrosarcoma think a chromosomal change or certain genetic mutations that aren’t part of an inherited disorder may change cartilage cells.

What are the complications of this condition?

Fast-growing chondrosarcomas like mesenchymal chondrosarcoma or dedifferentiated chondrosarcoma can be life-threatening because they’re quick to spread and affect other areas of your body.

How are chondrosarcomas diagnosed?

A healthcare provider will ask questions about your symptoms, including:

• When you first noticed them.
• If they’ve gotten worse.
• How they’ve affected your daily life.

They’ll do a physical examination and may order imaging tests or a biopsy.

Imaging tests

To diagnose chondrosarcomas, healthcare providers may order the following imaging tests:

• X-ray.
• Computed tomography (CT) scan.
• Magnetic resonance imaging (MRI) scan.
• Positron emission tomography (PET) scan.
• Bone scan.

Biopsy

Your provider may refer you to an orthopaedist, a surgeon who diagnoses and treats conditions including bone cancer, to do a biopsy. Your orthopaedist may do different types of biopsies to remove tissue from a suspicious lump so a pathologist can examine the tissue under a microscope. Those types are:

• Fine-needle aspiration: An orthopaedist uses a needle and syringe to get the tissue sample.
• Incisional biopsy: They cut into the tumor to remove a tissue sample.
• Excisional biopsy: They remove the entire lump or section of tissue.

A pathologist will examine the tissue sample for signs of cancerous cells. If cells are cancerous, they’ll identify the tumor type and establish a tumor grade.

What are the grades of chondrosarcomas?

A tumor grade is a measure of how fast a tumor may grow and spread. Chondrosarcoma grades range from 1 (I) to 3 (III):

• Low-grade (grade I) chondrosarcomas: Also called atypical cartilaginous tumors, these tend to grow the slowest and are very unlikely to spread.
• Intermediate-grade (grade II) chondrosarcomas: These are slightly more likely to spread.
• High-grade (grade III) chondrosarcomas: These are the most likely to spread.

What are treatments for chondrosarcoma?

Most of the time, you’ll need surgery to remove the chondrosarcoma. The specific surgery will depend on your situation, including what kind of chondrosarcoma you have, the tumor location and its grade, meaning how quickly it may spread. Surgeries for chondrosarcoma include:

• Curettage: In this procedure, your provider scrapes the tumor tissue from your bone and replaces the missing bone with bone graft material.
• Limb-sparing surgery: Your provider removes the tumor and nearby bone and muscle, followed by reconstructive surgery to replace the missing bone and tissue.

Very rarely, your orthopaedist may recommend surgery to remove part of your arm or leg (amputation). They may recommend you have a prosthetic limb. In all cases, you’ll have physical therapy to help you build strength, balance and mobility as you adapt to your situation.

If you have a rare type of chondrosarcoma like dedifferentiated or mesenchymal chondrosarcoma, you may have chemotherapy or radiation therapy before surgery to shrink the tumor, as well as after the surgery to kill any remaining cancer cells.

Can chondrosarcomas be prevented?

No, they can’t, partly because researchers don’t know exactly why they happen. Research shows people with certain inherited disorders have an increased risk of developing chondrosarcoma. Talk to a healthcare provider about your family medical history so they can assess your risk and recommend steps to monitor your health.

What can I expect if I have this condition?

There are several types of chondrosarcomas, each with different prognoses or expected outcomes. Ask your healthcare provider what you can expect given your specific situation. They’ll be glad to explain your prognosis.

Is there a cure for chondrosarcoma?

In some cases, surgery to remove very small, slow-growing conventional chondrosarcoma tumors may cure the condition. Based on the type of chondrosarcoma, adding chemotherapy may help cure the condition.

What are chondrosarcoma survival rates?

Survival rates for chondrosarcoma vary depending on the tumor type and whether the tumor is spreading to other parts of your body. Overall, 79% of people with chondrosarcoma were alive five years after diagnosis. Here are specific survival rates based on tumor location:

As you think about your situation, try to keep a few things in mind:

• A survival rate is an estimate, not a prediction. They’re estimates based on the experiences of other people, and your situation may be very different.
• Survival rate estimates reflect what happened in the past. For example, the survival rates listed above are based on what happened to people with chondrosarcoma between 2012 and 2018.
• Survival rates don’t indicate how long you’ll live.

If you have questions or concerns about what a survival rate estimate means in your case, ask your provider to explain how these estimates factor into your situation.

I have chondrosarcoma. How do I take care of myself?

Living with chondrosarcoma usually starts with recovering from treatment. Chondrosarcoma is often treated with surgery that might involve removing a limb or section of a limb to remove your cancer. Once you’ve recovered from surgery, you’ll need to start a rehabilitation program to help you adjust to using a prosthetic limb.

You might need help adjusting to other changes, such as how you go about your everyday life or changes in your appearance. Your healthcare provider will have suggestions for physical, occupational or mental health therapies that may help you manage these challenges.

When should I see my healthcare provider?

Ask your provider to explain the kinds of changes in your body that may be signs that cancer has come back, and when it makes sense for you to contact them.

What questions should I ask my healthcare provider about my diagnosis?

Here are some questions you might want to ask your healthcare provider:

• What kind of chondrosarcoma do I have?
• What treatments do you recommend?
• Why do you recommend those treatments?
• What are the treatment side effects?
• What are the outcomes of this treatment?
• If I have the recommended treatments, can my chondrosarcoma come back?

What is the difference between chondrosarcoma and osteosarcoma?

Osteosarcoma starts in the bone and typically affects children. Chondrosarcoma starts in your cartilage and typically affects adults. Healthcare providers may treat chondrosarcoma with surgery alone and treat osteosarcoma with chemotherapy and surgery.